Sanfordherrera4489

Although the overall number of organs involved was not related to OS, the number of organs involved in the heart, liver and kidney was related. Multivariate analysis indicated that cardiac involvement and negative hematologic response with persistence of positive immunofixation were independent prognostic factors for OS.

Cardiac involvement and the hematologic response to treatment were independent prognostic factors for OS in light-chain amyloidosis patients.

Cardiac involvement and the hematologic response to treatment were independent prognostic factors for OS in light-chain amyloidosis patients.The aim of this article is to assess the prevalence and severity of myocardial iron overload in thalassemia patients who present for the first time for cardiac MRI and to define the right age to start screening, in a resource-constrained environment. All MRI scans done at our institute for myocardial iron overload assessment in thalassemia patients, between 2015 and 2018 were analysed. check details Patients up to the age of 30 years were included. There were a total of 600 patients, (Age group between 2 and 30 years). All these scans were retrospectively analysed and severity of myocardial iron overload was categorized as normal, mild, moderate and severe based on the bright blood T2* equivalent values at 1.5 T. Overall prevalence of myocardial iron overload was 32.3%, while the prevalence of myocardial iron overload in patients in the age group 0-10 was 10.2%.There were 40 patients in the age group 0-6 years, of whom, only 2 had myocardial iron overload. In patients less than or equal to 6 years of age, the number of patients with iron overload was very small and this may be used to decide the optimal age for scanning.

High-altitude polycythemia (HAPC) is characterized by excessive proliferation of erythrocytes, resulting from the hypobaric hypoxia condition in high altitude. The genetic variants and molecular mechanisms of HAPC remain unclear in highlanders. We recruited 141 Tibetan dwellers, including 70 HAPC patients and 71 healthy controls, to detect the possible genetic variants associated with the disease; and performed targeted sequencing on 529 genes associated with the oxygen metabolism and erythrocyte regulation, utilized unconditional logistic regression analysis and GO (gene ontology) analysis to investigate the genetic variations of HAPC. We identified 12 single nucleotide variants, harbored in 12 genes, associated with the risk of HAPC (4.7 ≤ odd ratios ≤ 13.6; 7.6E - 08 ≤ 

-value ≤ 1E - 04). The pathway enrichment study of these genes indicated the three pathways, the PI3K-AKT pathway, JAK-STAT pathway, and HIF-1 pathway, are essential, which

-values as 3.70E - 08, 1.28 E - 07, and 3.98 E - 06, respectively. We are hopeful that our results will provide a reference for the etiology research of HAPC. However, additional genetic risk factors and functional investigations are necessary to confirm our results further.

The online version contains supplementary material available at 10.1007/s12288-021-01474-1.

The online version contains supplementary material available at 10.1007/s12288-021-01474-1.Radiotherapy is not usually a part of standard Burkitt lymphoma treatment. We aim to assess patient and treatment characteristics of Burkitt lymphoma, particularly RT use, and how they relate to survival. Retrospective cohort of adult patients treated from 2008 to 2019 in an academic hospital. All patients had biopsy-proven Burkitt's lymphoma staged I to IV according to St. Jude's/Murphy staging system. Patients were followed for at least six-months or until death. Forty-eight consecutive patients were selected. Median age at diagnosis was 36.9 years (18-62). Median follow-up was 7.78 months (0.5-187.5). Most were male (81.3%) and had good performance by ECOG scale on their first hematologist appointment (56.2% were ECOG 0). Median OS and PFS were 8.4 months (interquartile range Q1-Q3 3.96-152.2) and 8.3 months (interquartile range Q1-Q3 6.7-not reached), respectively, with 32 deaths. A total of 43 patients (89.6%) were HIV-positive and had a median CD4 + level of 193.5 cells/mm3 at diagnosis. Patients that did not present a drop in CD4 + levels after treatment had better OS than those that did (p = 0.020). 11 patients underwent radiotherapy (22.9%) who had better OS than those who did not (p = 0.015). Our findings show that adult patients living with HIV presenting Burkitt lymphoma who maintained their immune status throughout treatment had better prognosis than those who presented CD4 + cells drops. Also, patients treated with radiotherapy-either with palliative intent or as consolidation after chemotherapy-had statistically significant better OS than those not irradiated. Prospective data is warranted for radiotherapy as a consolidative and as a palliative treatment.In patients with Cerebral Venous Thrombosis (CVT), inherited and acquired thrombophilic conditions have been studied either individually or as subset of a comprehensive evaluation. None of the studies have included a comprehensive evaluation of all the known associations. The associations for various conditions have been found to differ significantly between the Indian and the Western population. We defined a Comprehensive Thrombophilia panel (inherited and acquired) comprising of 13 thrombophilic conditions to include all the relevant known associations in CVT. All patients in this cross-sectional study were evaluated as per the defined protocol during the three-year study period. We evaluated 42 patients of CVT for presence of inherited and acquired thrombophilic conditions. The mean age of the study population was 38.4 yrs. An inherited or an acquired thrombophilic condition was diagnosed in 76% patients. Hyperhomocysteinemia and raised factor VIII levels were the most common conditions, seen in 38% and 35.7% patients respectively. MTHFR mutation was seen in 21% patients. Protein S deficiency was seen in 7% patients. Factor V Leiden and JAK2 positive MPN were seen in 2.3% cases. We did not detect any patients with Protein C deficiency, APLA syndrome, anti-thrombin deficiency, PG20210A mutation or PNH. PAI-1 polymorphism was not included in the protocol as its role is controversial and it has not been established in Indian studies. There is an urgent need for Comprehensive Thrombophilia testing in a larger population of CVT patients to better delineate the spectrum of associated thrombophilic conditions. Such a study is bound to impact therapy and prognosis of CVT.Purpose Nivolumab is an anti-programmed cell death protein 1 (PD1) monoclonal antibody that is indicated in relapsed/refractory Hodgkin lymphoma (R/R HL) after autologous stem cell transplant (autoSCT). Purpose of our retrospective study was to assess safety and efficacy of Nivolumab in R/R HL as a bridge to autoSCT in patients who are refractory to ≥ 2 lines of chemotherapy. Methods Demographic data, number of chemotherapy regimens given previously, number of Nivolumab doses taken, and disease status on PET/CT were noted. Nivolumab was administered as a 3 mg/kg IV infusion every 2 weeks. The immunotherapy related adverse events (irAEs) were noted if any and documented. Results A total of 16 patients were included in the study. Ten patients were male and 6 were female. Median age was 22 years (range 3-32 years). The median number of treatment lines prior to Nivolumab was 3 (range 2-7). Nine patients had Complete Response (CR), 3 had Partial response (PR), 2 had Stable Disease (SD), 1 patient had pseudo-progression; classified as IR (3) and 1 expired before end of treatment evaluation. The drug was well tolerated, with mild irAEs noted. Twelve patients (75%) successfully underwent autoSCT. At a median follow up of 17.5 months (range 0.5-35 months), the progression- free survival (PFS) was 75% and overall survival (OS) was 87.5%. Conclusion Nivolumab is effective and safe in patients with R/R HL and is a good bridging therapy to autoSCT.Transfusion of RhD positive red cells to RhD negative individuals is not routine transfusion practice for the fear of alloimmunization. Aim of this study was to prospectively evaluate rate of alloimmunization after transfusion of RhD positive red cells in RhD negative individuals and to assess delay in transfusion due to decision making. This was a prospective, observational study conducted from 2014 to 2018. All patients were followed up for a period of three months, at 3, 14, 45 and 90 days with antibody screening. In addition, patients who were immunosuppressed and alloimmunized were followed up at 6 months and one year. During the period of the study, there were a total of 57 RhD negative patients (52 males and five females) who received a mean of 4.42 ± 2.85 transfusions. Alloimmunization was detected in 8 (14.03%) patients at a mean interval of 25.63 ± 16.04 days. Anti-D was detected in seven and one patient developed anti-E alloantibody. Mean number of red cell units transfused in alloimmunized was 1.7 ± 0.26 while it was 5.4 ± 1.82 in non-alloimmunized group. There was no delay in providing units to these patients. The TAT was found to be 68 min. Rate of alloimmunization after transfusion of RhD positive red cells to RhD negative individuals was found to be 12.3%. In life saving conditions, RhD negative patients can be transfused RhD positive red cells without delay in decision making.Immune thrombocytopenia (ITP) is a rare autoimmune disorder presenting with isolated thrombocytopenia. Splenectomy is still one of the treatment alternatives for these patients. Here we aim to analyze long term follow-up data of splenectomy in immune thrombocytopenia. This retrospectively designed study was conducted in a tertiary health clinic. Patients with ITP who were splenectomized between 1990 and 2015 were included. Response to treatment was interpreted as 'complete response', 'response' or 'no response'. The incidence of response loss was evaluated. Perioperative and long term complications and overall survival rates were determined. Out of 51 patients, who underwent splenectomy after 12 months of diagnosis, 47 achieved a response (92.2%). Of 47 patients who had a platelet count at least 30.000/µL, 41 (87.2%) had CR. Incidence of loss of response was 10.5% (95% confidence interval (CI) 4%-26.1%) at 30 months. Two patients died, and overall survival rate was 97.4% (95% CI 82.8%-99.6%) at 30 months of follow up. Considering the complications two patients had venous thromboembolism, 11 had minor bleeding episodes and 15 suffered from perioperative infections. Our study suggests that splenectomy promises a high level of response with acceptable complication rates. Although less preferred recently, splenectomy should still be taken into consideration when remission is not achieved especially after 12 months of disease.Gastric mucosa-associated lymphoid tissue non-Hodgkin lymphoma (gMALT NHL) is the second most common gastrointestinal lymphoma (50% of all gastric lymphomas), being closely associated with Helicobacter pylori infection, justifying that antibiotic therapy is effective in over 75% of all cases. This is a retrospective study analyzing all adult gMALT NHL cases diagnosed and treated in a single center for 8 years, focusing on demographic features, treatment outcomes, and survival analysis. Sixty patients with a median age of 61 years (53.3% female gender) were analyzed. Most of the cases had localized disease (66.7% were Lugano stage I) and had low IPI scores (median 1). There was a high prevalence of Helicobacter pylori infection (68.3%). Nearly 97% of the cases received treatment for the disease, a median of one line; 55% of the patients treated endured complete response after first-line therapy (mostly antibiotics). Median overall survival time and median progression-free survival time were not reached. The mean follow-up time was 81.